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Protein

Aconitate hydratase, mitochondrial

Gene

ACO2

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Catalyzes the isomerization of citrate to isocitrate via cis-aconitate.By similarity

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

[4Fe-4S] clusterBy similarityNote: Binds 1 [4Fe-4S] cluster per subunit. Binding of a [3Fe-4S] cluster leads to an inactive enzyme.By similarity

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: tricarboxylic acid cycle

This protein is involved in step 2 of the subpathway that synthesizes isocitrate from oxaloacetate.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Citrate synthase, mitochondrial (CS)
  2. Aconitate hydratase, mitochondrial (ACO2)
This subpathway is part of the pathway tricarboxylic acid cycle, which is itself part of Carbohydrate metabolism.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes isocitrate from oxaloacetate, the pathway tricarboxylic acid cycle and in Carbohydrate metabolism.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section describes the interaction between a single amino acid and another chemical entity. Priority is given to the annotation of physiological ligands.<p><a href='/help/binding' target='_top'>More...</a></p>Binding sitei99SubstrateBy similarity1
<p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi385Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi448Iron-sulfur (4Fe-4S)By similarity1
Metal bindingi451Iron-sulfur (4Fe-4S)By similarity1
Binding sitei474SubstrateBy similarity1
Binding sitei479SubstrateBy similarity1
Binding sitei607SubstrateBy similarity1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • 3 iron, 4 sulfur cluster binding Source: Ensembl
  • 4 iron, 4 sulfur cluster binding Source: GO_Central
  • aconitate hydratase activity Source: Reactome
  • iron ion binding Source: MGI

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionLyase
Biological processTricarboxylic acid cycle
Ligand4Fe-4S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

BioCyc Collection of Pathway/Genome Databases

More...
BioCyci
MetaCyc:HS02077-MONOMER

BRENDA Comprehensive Enzyme Information System

More...
BRENDAi
4.2.1.3 2681

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-1268020 Mitochondrial protein import
R-HSA-71403 Citric acid cycle (TCA cycle)

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...
UniPathwayi
UPA00223;UER00718

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Aconitate hydratase, mitochondrial (EC:4.2.1.3By similarity)
Short name:
Aconitase
Alternative name(s):
Citrate hydro-lyase
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:ACO2
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 22

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...
EuPathDBi
HostDB:ENSG00000100412.15

Human Gene Nomenclature Database

More...
HGNCi
HGNC:118 ACO2

Online Mendelian Inheritance in Man (OMIM)

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MIMi
100850 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_Q99798

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Mitochondrion

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Infantile cerebellar-retinal degeneration (ICRD)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA severe autosomal recessive neurodegenerative disorder characterized by onset between ages 2 and 6 months of truncal hypotonia, athetosis, seizures, and ophthalmologic abnormalities, particularly optic atrophy and retinal degeneration. Affected individuals show profound psychomotor retardation, with only some achieving rolling, sitting, or recognition of family. Brain MRI shows progressive cerebral and cerebellar degeneration.
See also OMIM:614559
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_067543112S → R in ICRD; functional expression studies in yeast show that the mutant has decreased function under growth conditions requiring the TCA cycle and the glyoxylate shunt. 1 PublicationCorresponds to variant dbSNP:rs786200924EnsemblClinVar.1
Natural variantiVAR_073436259G → D in ICRD. 1 PublicationCorresponds to variant dbSNP:rs786204828EnsemblClinVar.1
Natural variantiVAR_073438736K → N in ICRD. 1 PublicationCorresponds to variant dbSNP:rs786204829EnsemblClinVar.1
Optic atrophy 9 (OPA9)1 Publication
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA condition that features progressive visual loss in association with optic atrophy. Atrophy of the optic disk indicates a deficiency in the number of nerve fibers which arise in the retina and converge to form the optic disk, optic nerve, optic chiasm and optic tracts.
See also OMIM:616289
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07343574L → V in OPA9. 1 PublicationCorresponds to variant dbSNP:rs141772938EnsemblClinVar.1
Natural variantiVAR_073437661G → R in OPA9. 1 PublicationCorresponds to variant dbSNP:rs752034900EnsemblClinVar.1

Keywords - Diseasei

Disease mutation, Neurodegeneration

Organism-specific databases

DisGeNET

More...
DisGeNETi
50

MalaCards human disease database

More...
MalaCardsi
ACO2
MIMi614559 phenotype
616289 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000100412

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
98676 Autosomal recessive isolated optic atrophy
313850 Infantile cerebellar-retinal degeneration

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA24443

Chemistry databases

Drug and drug target database

More...
DrugBanki
DB03964 4-Hydroxy-Aconitate Ion
DB04351 Aconitate Ion
DB04072 Alpha-Methylisocitric Acid
DB01727 Isocitric Acid
DB04562 Tricarballylic Acid

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
ACO2

Domain mapping of disease mutations (DMDM)

More...
DMDMi
6686275

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 27MitochondrionBy similarityAdd BLAST27
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000000054128 – 780Aconitate hydratase, mitochondrialAdd BLAST753

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei31N6-succinyllysineBy similarity1
Modified residuei50N6-acetyllysine; alternateBy similarity1
Modified residuei50N6-succinyllysine; alternateBy similarity1
Modified residuei138N6-acetyllysine; alternateBy similarity1
Modified residuei138N6-succinyllysine; alternateBy similarity1
Modified residuei144N6-acetyllysine; alternateBy similarity1
Modified residuei144N6-succinyllysine; alternateBy similarity1
Modified residuei233N6-acetyllysine; alternateBy similarity1
Modified residuei233N6-succinyllysine; alternateBy similarity1
Modified residuei411N6-succinyllysineBy similarity1
Modified residuei549N6-succinyllysineBy similarity1
Modified residuei559PhosphoserineCombined sources1
Modified residuei573N6-acetyllysine; alternateCombined sources1
Modified residuei573N6-succinyllysine; alternateBy similarity1
Modified residuei577N6-succinyllysineBy similarity1
Modified residuei591N6-succinyllysineBy similarity1
Modified residuei605N6-acetyllysine; alternateCombined sources1
Modified residuei605N6-succinyllysine; alternateBy similarity1
Modified residuei628N6-succinyllysineBy similarity1
Modified residuei670PhosphoserineBy similarity1
Modified residuei689N6-succinyllysineBy similarity1
Modified residuei723N6-acetyllysine; alternateBy similarity1
Modified residuei723N6-succinyllysine; alternateBy similarity1
Modified residuei730N6-acetyllysine; alternateBy similarity1
Modified residuei730N6-succinyllysine; alternateBy similarity1
Modified residuei736N6-acetyllysineBy similarity1
Modified residuei739N6-acetyllysineBy similarity1
Modified residuei743N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

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EPDi
Q99798

MaxQB - The MaxQuant DataBase

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MaxQBi
Q99798

PaxDb, a database of protein abundance averages across all three domains of life

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PaxDbi
Q99798

PeptideAtlas

More...
PeptideAtlasi
Q99798

PRoteomics IDEntifications database

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PRIDEi
Q99798

ProteomicsDB human proteome resource

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ProteomicsDBi
78478

2D gel databases

DOSAC-COBS 2D-PAGE database

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DOSAC-COBS-2DPAGEi
Q99798

REPRODUCTION-2DPAGE

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REPRODUCTION-2DPAGEi
IPI00017855
Q99798

Two-dimensional polyacrylamide gel electrophoresis database from the Geneva University Hospital

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SWISS-2DPAGEi
Q99798

University College Dublin 2-DE Proteome Database

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UCD-2DPAGEi
Q99798

PTM databases

CarbonylDB database of protein carbonylation sites

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CarbonylDBi
Q99798

GlyConnect protein glycosylation platform

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GlyConnecti
988

iPTMnet integrated resource for PTMs in systems biology context

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iPTMneti
Q99798

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

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PhosphoSitePlusi
Q99798

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
Q99798

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

Gene expression databases

Bgee dataBase for Gene Expression Evolution

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Bgeei
ENSG00000100412 Expressed in 229 organ(s), highest expression level in heart right ventricle

CleanEx database of gene expression profiles

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CleanExi
HS_ACO2

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
Q99798 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
Q99798 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
HPA001097

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Monomer.By similarity

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

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BioGridi
106566, 31 interactors

Protein interaction database and analysis system

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IntActi
Q99798, 6 interactors

STRING: functional protein association networks

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STRINGi
9606.ENSP00000216254

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

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ProteinModelPortali
Q99798

SWISS-MODEL Repository - a database of annotated 3D protein structure models

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SMRi
Q99798

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni192 – 194Substrate bindingBy similarity3
Regioni670 – 671Substrate bindingBy similarity2

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the aconitase/IPM isomerase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG0453 Eukaryota
COG1048 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00940000154892

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

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HOGENOMi
HOG000224293

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG000248

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
Q99798

KEGG Orthology (KO)

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KOi
K01681

Database for complete collections of gene phylogenies

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PhylomeDBi
Q99798

TreeFam database of animal gene trees

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TreeFami
TF300627

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...
Gene3Di
3.20.19.10, 1 hit
3.30.499.10, 2 hits
3.40.1060.10, 1 hit

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR015931 Acnase/IPM_dHydase_lsu_aba_1/3
IPR001030 Acoase/IPM_deHydtase_lsu_aba
IPR015928 Aconitase/3IPM_dehydase_swvl
IPR018136 Aconitase_4Fe-4S_BS
IPR036008 Aconitase_4Fe-4S_dom
IPR015932 Aconitase_dom2
IPR006248 Aconitase_mito-like
IPR000573 AconitaseA/IPMdHydase_ssu_swvl

Pfam protein domain database

More...
Pfami
View protein in Pfam
PF00330 Aconitase, 1 hit
PF00694 Aconitase_C, 1 hit

Protein Motif fingerprint database; a protein domain database

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PRINTSi
PR00415 ACONITASE

Superfamily database of structural and functional annotation

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SUPFAMi
SSF53732 SSF53732, 1 hit

TIGRFAMs; a protein family database

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TIGRFAMsi
TIGR01340 aconitase_mito, 1 hit

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS00450 ACONITASE_1, 1 hit
PS01244 ACONITASE_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

Q99798-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAPYSLLVTR LQKALGVRQY HVASVLCQRA KVAMSHFEPN EYIHYDLLEK
60 70 80 90 100
NINIVRKRLN RPLTLSEKIV YGHLDDPASQ EIERGKSYLR LRPDRVAMQD
110 120 130 140 150
ATAQMAMLQF ISSGLSKVAV PSTIHCDHLI EAQVGGEKDL RRAKDINQEV
160 170 180 190 200
YNFLATAGAK YGVGFWKPGS GIIHQIILEN YAYPGVLLIG TDSHTPNGGG
210 220 230 240 250
LGGICIGVGG ADAVDVMAGI PWELKCPKVI GVKLTGSLSG WSSPKDVILK
260 270 280 290 300
VAGILTVKGG TGAIVEYHGP GVDSISCTGM ATICNMGAEI GATTSVFPYN
310 320 330 340 350
HRMKKYLSKT GREDIANLAD EFKDHLVPDP GCHYDQLIEI NLSELKPHIN
360 370 380 390 400
GPFTPDLAHP VAEVGKVAEK EGWPLDIRVG LIGSCTNSSY EDMGRSAAVA
410 420 430 440 450
KQALAHGLKC KSQFTITPGS EQIRATIERD GYAQILRDLG GIVLANACGP
460 470 480 490 500
CIGQWDRKDI KKGEKNTIVT SYNRNFTGRN DANPETHAFV TSPEIVTALA
510 520 530 540 550
IAGTLKFNPE TDYLTGTDGK KFRLEAPDAD ELPKGEFDPG QDTYQHPPKD
560 570 580 590 600
SSGQHVDVSP TSQRLQLLEP FDKWDGKDLE DLQILIKVKG KCTTDHISAA
610 620 630 640 650
GPWLKFRGHL DNISNNLLIG AINIENGKAN SVRNAVTQEF GPVPDTARYY
660 670 680 690 700
KKHGIRWVVI GDENYGEGSS REHAALEPRH LGGRAIITKS FARIHETNLK
710 720 730 740 750
KQGLLPLTFA DPADYNKIHP VDKLTIQGLK DFTPGKPLKC IIKHPNGTQE
760 770 780
TILLNHTFNE TQIEWFRAGS ALNRMKELQQ
Length:780
Mass (Da):85,425
Last modified:May 30, 2000 - v2
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i58C9FFBDBDC63D5E
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
A2A274A2A274_HUMAN
Aconitate hydratase, mitochondrial
ACO2
805Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti35S → T in AAB38416 (Ref. 1) Curated1
Sequence conflicti136G → D in AAB38416 (Ref. 1) Curated1
Sequence conflicti159A → D in AAB38416 (Ref. 1) Curated1
Sequence conflicti167K → S in AAB38416 (Ref. 1) Curated1
Sequence conflicti199G → D in CAG38805 (Ref. 4) Curated1
Sequence conflicti207G → R in AAH26196 (PubMed:15489334).Curated1
Sequence conflicti242S → T in AAB38416 (Ref. 1) Curated1
Sequence conflicti270P → H in AAH26196 (PubMed:15489334).Curated1
Sequence conflicti275I → M in AAB38416 (Ref. 1) Curated1
Sequence conflicti444L → P in CAG38805 (Ref. 4) Curated1
Sequence conflicti517T → K in AAB38416 (Ref. 1) Curated1
Sequence conflicti553G → R in AAB38416 (Ref. 1) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07343574L → V in OPA9. 1 PublicationCorresponds to variant dbSNP:rs141772938EnsemblClinVar.1
Natural variantiVAR_067543112S → R in ICRD; functional expression studies in yeast show that the mutant has decreased function under growth conditions requiring the TCA cycle and the glyoxylate shunt. 1 PublicationCorresponds to variant dbSNP:rs786200924EnsemblClinVar.1
Natural variantiVAR_073436259G → D in ICRD. 1 PublicationCorresponds to variant dbSNP:rs786204828EnsemblClinVar.1
Natural variantiVAR_073437661G → R in OPA9. 1 PublicationCorresponds to variant dbSNP:rs752034900EnsemblClinVar.1
Natural variantiVAR_036572697T → N in a breast cancer sample; somatic mutation. 1 Publication1
Natural variantiVAR_073438736K → N in ICRD. 1 PublicationCorresponds to variant dbSNP:rs786204829EnsemblClinVar.1
Natural variantiVAR_033297768A → S. Corresponds to variant dbSNP:rs1804785Ensembl.1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

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DDBJi
Links Updated
U80040 mRNA Translation: AAB38416.1
U87939
, U87926, U87927, U87928, U87929, U87930, U87931, U87932, U87933, U87934, U87935, U87936, U87937, U87938 Genomic DNA Translation: AAC39921.1
CR456365 mRNA Translation: CAG30251.1
CR536568 mRNA Translation: CAG38805.1
AL023553 Genomic DNA No translation available.
AL008582 Genomic DNA No translation available.
BC014092 mRNA Translation: AAH14092.1
BC026196 mRNA Translation: AAH26196.1

The Consensus CDS (CCDS) project

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CCDSi
CCDS14017.1

Protein sequence database of the Protein Information Resource

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PIRi
S17526
T52543

NCBI Reference Sequences

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RefSeqi
NP_001089.1, NM_001098.2

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.643610

Genome annotation databases

Ensembl eukaryotic genome annotation project

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Ensembli
ENST00000216254; ENSP00000216254; ENSG00000100412

Database of genes from NCBI RefSeq genomes

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GeneIDi
50

KEGG: Kyoto Encyclopedia of Genes and Genomes

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KEGGi
hsa:50

UCSC genome browser

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UCSCi
uc003bac.3 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Wikipedia

Aconitase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U80040 mRNA Translation: AAB38416.1
U87939
, U87926, U87927, U87928, U87929, U87930, U87931, U87932, U87933, U87934, U87935, U87936, U87937, U87938 Genomic DNA Translation: AAC39921.1
CR456365 mRNA Translation: CAG30251.1
CR536568 mRNA Translation: CAG38805.1
AL023553 Genomic DNA No translation available.
AL008582 Genomic DNA No translation available.
BC014092 mRNA Translation: AAH14092.1
BC026196 mRNA Translation: AAH26196.1
CCDSiCCDS14017.1
PIRiS17526
T52543
RefSeqiNP_001089.1, NM_001098.2
UniGeneiHs.643610

3D structure databases

ProteinModelPortaliQ99798
SMRiQ99798
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi106566, 31 interactors
IntActiQ99798, 6 interactors
STRINGi9606.ENSP00000216254

Chemistry databases

DrugBankiDB03964 4-Hydroxy-Aconitate Ion
DB04351 Aconitate Ion
DB04072 Alpha-Methylisocitric Acid
DB01727 Isocitric Acid
DB04562 Tricarballylic Acid

PTM databases

CarbonylDBiQ99798
GlyConnecti988
iPTMnetiQ99798
PhosphoSitePlusiQ99798
SwissPalmiQ99798

Polymorphism and mutation databases

BioMutaiACO2
DMDMi6686275

2D gel databases

DOSAC-COBS-2DPAGEiQ99798
REPRODUCTION-2DPAGEiIPI00017855
Q99798
SWISS-2DPAGEiQ99798
UCD-2DPAGEiQ99798

Proteomic databases

EPDiQ99798
MaxQBiQ99798
PaxDbiQ99798
PeptideAtlasiQ99798
PRIDEiQ99798
ProteomicsDBi78478

Protocols and materials databases

The DNASU plasmid repository

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DNASUi
50
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000216254; ENSP00000216254; ENSG00000100412
GeneIDi50
KEGGihsa:50
UCSCiuc003bac.3 human

Organism-specific databases

Comparative Toxicogenomics Database

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CTDi
50
DisGeNETi50
EuPathDBiHostDB:ENSG00000100412.15

GeneCards: human genes, protein and diseases

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GeneCardsi
ACO2
HGNCiHGNC:118 ACO2
HPAiHPA001097
MalaCardsiACO2
MIMi100850 gene
614559 phenotype
616289 phenotype
neXtProtiNX_Q99798
OpenTargetsiENSG00000100412
Orphaneti98676 Autosomal recessive isolated optic atrophy
313850 Infantile cerebellar-retinal degeneration
PharmGKBiPA24443

GenAtlas: human gene database

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GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG0453 Eukaryota
COG1048 LUCA
GeneTreeiENSGT00940000154892
HOGENOMiHOG000224293
HOVERGENiHBG000248
InParanoidiQ99798
KOiK01681
PhylomeDBiQ99798
TreeFamiTF300627

Enzyme and pathway databases

UniPathwayi
UPA00223;UER00718

BioCyciMetaCyc:HS02077-MONOMER
BRENDAi4.2.1.3 2681
ReactomeiR-HSA-1268020 Mitochondrial protein import
R-HSA-71403 Citric acid cycle (TCA cycle)

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

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ChiTaRSi
ACO2 human

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

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GenomeRNAii
50

Protein Ontology

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PROi
PR:Q99798

The Stanford Online Universal Resource for Clones and ESTs

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SOURCEi
Search...

Gene expression databases

BgeeiENSG00000100412 Expressed in 229 organ(s), highest expression level in heart right ventricle
CleanExiHS_ACO2
ExpressionAtlasiQ99798 baseline and differential
GenevisibleiQ99798 HS

Family and domain databases

Gene3Di3.20.19.10, 1 hit
3.30.499.10, 2 hits
3.40.1060.10, 1 hit
InterProiView protein in InterPro
IPR015931 Acnase/IPM_dHydase_lsu_aba_1/3
IPR001030 Acoase/IPM_deHydtase_lsu_aba
IPR015928 Aconitase/3IPM_dehydase_swvl
IPR018136 Aconitase_4Fe-4S_BS
IPR036008 Aconitase_4Fe-4S_dom
IPR015932 Aconitase_dom2
IPR006248 Aconitase_mito-like
IPR000573 AconitaseA/IPMdHydase_ssu_swvl
PfamiView protein in Pfam
PF00330 Aconitase, 1 hit
PF00694 Aconitase_C, 1 hit
PRINTSiPR00415 ACONITASE
SUPFAMiSSF53732 SSF53732, 1 hit
TIGRFAMsiTIGR01340 aconitase_mito, 1 hit
PROSITEiView protein in PROSITE
PS00450 ACONITASE_1, 1 hit
PS01244 ACONITASE_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

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ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiACON_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: Q99798
Secondary accession number(s): O75809
, Q5JZ41, Q6FHX0, Q8TAQ6
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1997
Last sequence update: May 30, 2000
Last modified: December 5, 2018
This is version 189 of the entry and version 2 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human chromosome 22
    Human chromosome 22: entries, gene names and cross-references to MIM
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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